FACT SHEET: Ehlers-Danlos Syndromes1 (also known as “Ehlers Danlos Syndrome” and “EDS”; includes, in descending order of prevalence, “hypermobile EDS” [hEDS]), “classical EDS [cEDS]; “vascular EDS” [vEDS]; and “arthrochalasia EDS” [aEDS]; more rare2 types include “periodontal EDS” [pEDS], “cardiac-valvular EDS” [cvEDS]; “kyphotic sclerotic EDS” [kEDS], “spondyloplastic EDS” [spEDS], “brittle cornea syndrome” [BCS], “musculocontracture EDS” [mcEDS], “classical-like EDS” [clEDS], “dermatopraxis EDS” [dEDS], and “myopathic EDS” [mEDS].)
Is the initiation of non-invasive dental hygiene procedures* contra-indicated?
- No, unless there are medical issues that may render the procedures potentially unsafe (e.g., pneumothorax and craniocervical/atlantoaxial instability).
Is medical consult advised?
- Yes, liaison with the patient/client’s EDS specialist/most responsible physician is advisable regarding the type, severity, and management of the patient/client’s EDS before undertaking dental hygiene treatment for the first time. (This includes bleeding risk, if any, for fitting a mouth guard and taking an impression.) Consult is also warranted if patient/client’s disease seems not to be optimally medically managed3, as well as for assessment of cervical spine instability.
Is the initiation of invasive dental hygiene procedures contra-indicated?**
- Yes. EDS encompasses elements of a blood/bleeding disorder4 that may affect appropriateness or safety. Scaling and root planing, including curetting of surrounding tissue, are contraindicated until the patient/client is medically cleared. In some cases, antibiotic prophylaxis is also required. As well, certain medical complications may render the procedures potentially unsafe (e.g., pneumothorax and craniocervical/atlantoaxial instability).
Is medical consult advised?
- See above.
Is medical clearance required?
Is antibiotic prophylaxis required?
- No, in most cases. However, certain types of EDS (in particular cardiac-valvular EDS) cause cardiac valve abnormalities that may necessitate prosthetic valve placement. Antibiotic prophylaxis for infective endocarditis should be administered in accordance with current guidelines of the American Heart Association. (Refer to the Infective Endocarditis Fact Sheet and Advisory in the Knowledge Network Library for more detailed information.)
Is postponing treatment advised?
- Possibly, but not typically (depends on type, severity, and management of the disease, as well as medical clearance for bleeding predisposition).
Oral management implications
- Oral and mandibular signs/symptoms of EDS are under-recognized by healthcare practitioners, meaning that the dental hygienist plays an important role in identification of dysfunction. Because of EDS’s hereditary pattern, the dental hygienist should be alert for signs/symptoms of EDS in relatives of their affected patient/client.
- While EDS can result in significant effects on oral health and mouth function, for the majority of EDS patients/clients (most of whom have hEDS), the direct impact on the dental hygiene visit is likely to be modest. Some types or manifestations of EDS, however, pose significant management challenges for the dental hygienist.
- The cardiovascular status of all patients/clients should be evaluated and monitored. Dysautonomia can result in orthostatic hypotension and postural tachycardia, and the dental hygienist should take this into account for positional changes in affected EDS patients/clients (specifically, the transition from reclining/sitting to standing up). Similarly, the dental hygienist should be alert for “allergy-like” manifestations of mast cell disease.
- Craniocervical instability (CCI) and atlantoaxial instability (AAI) are potential concerns in patients/clients with EDS. In affected patients/clients, appropriate precautions should be taken to ensure safe dental hygiene practice (e.g., minimization of C-spine movement).
- Shorter appointments may decrease the risk of temporomandibular joint (TMJ) dislocation and pain. A bite block and frequent breaks are also considerations.
- Neck and lumbar support in the dental chair may be required.
- Some patients/clients may have difficulty walking, be prone to falling over, and have poor body control.
- The oral manifestations of EDS (including delicate mucosa and weakened gingival tissue) may result in significant bleeding on probing, scaling, and other dental procedures/examinations. Thin gingival tissue can be easily torn during scaling procedures.
- Poor wound healing and excessive bleeding are common with oral procedures, as well as with other trauma. Stitches often tear out.
- Resistance to local anaesthetics may occur.5
- Prevention of tooth decay and gum disease are very important for patients/clients with EDS. This will reduce the need for tooth extractions and complex dental procedures, for which EDS patients/clients are at elevated risk of complications. A low cariogenic diet should be recommended, and a high standard of oral hygiene needs to be maintained. Fluoride application and dietitian referral should be considered by the dental hygienist.
- Ultra-soft toothbrushes should be used to minimize damage to the gingival margin. Similarly, floss needs to be used carefully to avoid traumatizing friable gingiva.
- Measures to reduce the amplification effects of teeth grinding and jaw clenching are desirable. EDS patients/clients are already predisposed to temporomandibular disorder (TMD).
- Posture, neck, and upper back issues should be addressed, because of the interplay between these and TMD. TMJ-protective and TMD management measures may also entail lifestyle changes such as soft diet and altered chewing patterns; avoidance of wide or straining jaw movements; application of ice packs to reduce pain; stress reduction techniques; and physical activity management.
- Reduction of muscle spasms and diminution of TMD pain may be accomplished with use, under medical supervision, of muscle relaxants, analgesics, anti-inflammatories, and antidepressants. Botulinum toxin may be consideration for some patients/clients. Physical therapy may be helpful.
- Due to accelerated tooth movement, orthodontic treatment may need to be completed in one year or less. However, relapse is common, and tooth movement is usually noted within 18 months post-treatment. Protective wax over braces may lessen the risk of mouth ulcers.
- Mouth and jaw structures are affected differently in the various types of EDS, with oral and mandibular signs/symptoms being reported in all types.
- Collagen defects adversely affect blood vessels, gingiva, bone, teeth, and nerve tissue, in addition to the ligaments and tendons that hold jaw and facial structures in position.
- Orofacial features in some patients/clients include a narrow maxilla, wide nasal bridge, and flattened midface. Micrognathia (small mandible) may be present.
- The mucosa of the mouth is often thin and fragile, leading to ulcers and easy wounding with oral appliances, as well as bleeding.
- Absence of frenula (lingual and/or labial) is seen in some patients/clients.6
- Tooth anomalies include: high cusps and deep anatomic grooves in molars and premolars; roots that are abnormally shaped/dilacerated, elongated, and/or joined; teeth that are absent or poorly formed; enamel hypoplasia and enamel/dentine defects; and free-floating coronal pulp stones7.
- Excessive tooth movement occurs, which may accelerate gingival recession.
- Risk of caries is elevated in teeth that have enamel defects. Pulpitis, abscess formation, and tooth loss are other sequelae.
- Multiple odontogenic keratocysts8 can cause local bony destruction of the jaws in vEDS.
- Early onset of gingivitis and periodontitis occur in pEDS. Severe periodontal disease leads to early tooth loss by the second or third decade of life. To a lesser degree, periodontal disease may occur at elevated rates in cEDS and vEDS.
- Halitosis (bad breath) and altered taste may result from gingival and periodontal disease.
- Temporomandibular disorder9 is common. The temporomandibular joint (TMJ) is typically hypermobile, and it is prone to dislocation. TMJ relocation from hyperextension is associated with pain and tissue/joint damage, which ultimately may limit mobility following repeated episodes.
- Muscles of chewing may be overused, and they are prone to spasm10.
- Increased pharyngeal collapsibility can result from tissue flaccidity.
Related signs and symptoms
- The Ehlers-Danlos syndromes comprise 13 genetic, hereditary connective tissue disorders. In all types, structural collagen and its function are altered, leading to joint hypermobility, skin hyperextensibility, and tissue fragility of varying degrees. Other signs/symptoms are only observed in specific types of EDS. Severe cardiovascular, pulmonary, and gastrointestinal complications may occur. Even within the same type of EDS, manifestations may vary substantially.
- With an overall prevalence of at least 1/5,000, EDS affects some 3,000 persons in Ontario (7,900 in Canada).
- Overly flexible joints lead to joint pain, subluxation, and even spontaneous dislocation. Extreme joint laxity leads to flat feet, genu recurvarum (back knee), kyphoscoliosis, and other skeletal deformities.
- Weakened connective tissue leads to stretchy skin that tends to snap back into place when released after pulling.
- Fragile skin and tissue result in poor healing of wounds, defective scarring, and easy bruising. Other cutaneous manifestations include molluscoid pseudotumours11, subcutaneous lipid-containing cysts (which may calcify), and redundant skin on the palms and soles.
- Most patients/clients tend to bleed more than normal. This ranges from mild bruising (such as in most hEDS and cEDS) to severe hematoma formation and bleeding from the nose, gut, lungs, and urogenital tract (such as in vEDS).
- Craniocervical/atlantloaxial instability12 may present with neck, shoulder, and/or jaw pain. Other warning signs/symptoms include severe headaches, dysthesias13, tunnel vision, nausea, tinnitus (ringing in the ears), and cognition issues.
- Pain and fatigue characterize all types of EDS, and muscle weakness is common.
- Epicanthic folds (which extend from the nasal bridge to the upper eyelids) can give the appearance of wideset eyes (hypertelorism) and widened nasal bridge. Other ocular features include: prominent or puffy upper eyelids; bluish sclera (in cEDS, aEDS, and kEDS); ability to evert the upper eyelid (cEDS); myopia (short-sightedness); strabismus (ocular misalignment; in cEDS); prominent, “staring” eyes (due to lack of subcutaneous tissue; in vEDS); and down-staring palpebral fissures (in kEDS).
- The nose may appear pinched or sharp (vEDS), or the bridge of the nose can be flattened or widened (cEDS and kEDS).
- Café-au-lait (brown) spots may be present in some types of EDS, particularly on the skin of the neck.
- While affected persons often first become symptomatic in their early twenties, overt clinical manifestations may first arise in childhood. These childhood findings include orthostatic intolerance, urinary incontinence, diarrhea, and poor postural control.
- Hypermobile EDS (autosomal dominant, and accounting for 80% to 90% of EDS cases) is characterized by generalized joint hypermobility and instability, as well as chronic pain. Shoulder dislocation is often the first sign of EDS.
- Classical EDS (autosomal dominant) is characterized by extremely stretchy skin with a doughy or velvety texture, as well as skin fragility with atrophic scarring. “Cigarette paper” scar formation is particularly evident on the face, forehead, lower legs, and over pressure points.
- Vascular EDS (autosomal dominant) is characterized by extensive bruising, arterial fragility with aneurysm/dissection/ rupture, organ fragility and rupture (including of the intestines), and pneumothorax14. Rupture of the aorta is usually fatal, and potential rupture of the uterus is a particular concern during pregnancy. There may also be lack of earlobes, and the pinna of the ears may be firm. Patients/clients often have an older facial appearance, with sharpened nose, thin lips, hollow cheeks, and prominent eyes.
- Arthrochalasia EDS (autosomal dominant) is characterized by severe joint hypermobility and congenital bilateral dislocation of the hips.
- Periodontal EDS (autosomal recessive) is characterized by pretibial plaques (discolouration of shins), in addition to oral manifestations.
- Cardiac-valvular EDS (autosomal recessive) is characterized by severe heart valve insufficiency.
- Dysautonomia15 and mast cell diseases16 often occur in persons living with EDS.
- Obstructive sleep apnea (OSA) has been linked to EDS17, which can contribute to fatigue, excessive daytime sleepiness, and reduced quality of life.
- Other sequelae of EDS include bladder dysfunction (such as overactive bladder, incontinence, and urinary retention), hernias, visceral prolapse/ptosis18, neuropathies, and scoliosis.
- Osteoarthritis is a frequent complication of recurrent joint dislocations.
- Anxiety and depression are frequent co-morbidities.
- Sudden death in adolescence or early adult life may occur as a result of ruptured arteries and dissecting aneurysms, particularly in vEDS.
References and sources of more detailed information
- College of Dental Hygienists of Ontario
- Lepperdinger U, Zschocke J, Kapferer-Seebacher I. Oral manifestations of Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2021 Dec;187(4):520-526. doi: 10.1002/ajmg.c.31941. Epub 2021 Nov 6. PMID: 34741498; PMCID: PMC9298068. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9298068/
- Giroux CM, Carter L and Corkett J. An Exploration of Quality of Life among Ontario Postsecondary Students Living with the Chronic Illness Ehlers-Danlos Syndrome. The Canadian Journal for the Scholarship of Teaching and Learning. 2020 Jun;11(1). doi: https://doi.org/10.5206/cjsotl-rcacea.2020.1.10766.
- Zhou Z, Rewari A, Shanthanna H. Management of chronic pain in Ehlers-Danlos syndrome: Two case reports and a review of literature. Medicine (Baltimore). 2018 Nov;97(45):e13115. doi: 10.1097/MD.0000000000013115. PMID: 30407326; PMCID: PMC6250522. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6250522/
- Busch A, Hoffjan S, Bergmann F, Hartung B, Jung H, Hanel D, Tzschach A, Kadar J, von Kodolitsch Y, Germer CT, Trobisch H, Strasser E, Wildenauer R. Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration. Orphanet J Rare Dis. 2016 Aug 3;11(1):111. doi: 10.1186/s13023-016-0491-2. PMID: 27488172; PMCID: PMC4971646. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4971646/
- Artoni A, Bassotti A, Abbattista M, Marinelli B, Lecchi A, Gianniello F, Clerici M, Bucciarelli P, La Marca S, Peyvandi F, Martinelli I. Hemostatic abnormalities in patients with Ehlers-Danlos syndrome. J Thromb Haemost. 2018 Dec;16(12):2425-2431. doi: 10.1111/jth.14310. Epub 2018 Nov 8. PMID: 30312027. https://onlinelibrary.wiley.com/doi/10.1111/jth.14310
- Gaisl T, Giunta C, Bratton DJ, et al. Obstructive sleep apnoea and quality of life in Ehlers-Danlos syndrome: a parallel cohort study Thorax 2017;72:729-735. https://thorax.bmj.com/content/72/8/729
- Sedky K, Gaisl T, Bennett DS. Prevalence of obstructive sleep apnea in joint hypermobility syndrome: a systematic review and meta-analysis. J Clin Sleep Med. 2019;15(2):293–299. https://jcsm.aasm.org/doi/full/10.5664/jcsm.7636
- Chango Azanza DX, Munín MA, Sánchez GA, Spernanzoni F. Prolapse and regurgitation of the four heart valves in a patient with Ehlers-Danlos Syndrome: a case report. Eur Heart J Case Rep. 2019 Jun 1;3(2):ytz052. doi: 10.1093/ehjcr/ytz052. PMID: 31449614; PMCID: PMC6601149. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6601149/
- Henderson FC Sr, Rosenbaum R, Narayanan M, Koby M, Tuchman K, Rowe PC, Francomano C. Atlanto-axial rotary instability (Fielding type 1): characteristic clinical and radiological findings, and treatment outcomes following alignment, fusion, and stabilization. Neurosurg Rev. 2021 Jun;44(3):1553-1568. doi: 10.1007/s10143-020-01345-9. Epub 2020 Jul 4. PMID: 32623537; PMCID: PMC8121728. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8121728/
- Maini K, Dua A. Temporomandibular Syndrome. [Updated 2022 Apr 28]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. https://www.ncbi.nlm.nih.gov/books/NBK551612/
- Borghesi A, Nardi C, Giannitto C, Tironi A, Maroldi R, Di Bartolomeo F, Preda L. Odontogenic keratocyst: imaging features of a benign lesion with an aggressive behaviour. Insights Imaging. 2018 Oct;9(5):883-897. doi: 10.1007/s13244-018-0644-z. Epub 2018 Jul 31. PMID: 30066143; PMCID: PMC6206371. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6206371/
- Shumway NK, Cole E, Fernandez KH. Neurocutaneous disease: Neurocutaneous dysesthesias. J Am Acad Dermatol. 2016 Feb;74(2):215-28; quiz 229-30. doi: 10.1016/j.jaad.2015.04.059. PMID: 26775772.
- Dimensions of Dental Hygiene
- RDH Magazine
- GoodHope Ehlers-Danlos Syndrome Clinic, University Health Network
- Ehlers-Danlos Syndrome Canada
- The Ehlers-Danlos Society
- Mayo Clinic
- American Lung Association
- National Institute of Neurological Disorders and Stroke, National Institutes of Health
- Allergy & Asthma Network
- Ehlers Danlos Support UK
- Bowen DM (ed.) and Pieren JA (ed.). Darby and Walsh Dental Hygiene: Theory and Practice (5th edition). St. Louis: Elsevier; 2020.
- Little JW, Falace DA, Miller CS and Rhodus NL. Dental Management of the Medically Compromised Patient (9th edition). St. Louis: Elsevier Mosby; 2018.
- Ibsen OAC and Phelan JA. Oral Pathology For The Dental Hygienist (8th edition). St. Louis: Elsevier; 2023.
- Regezi JA, Sciubba JJ, and Jordan RCK. Oral Pathology: Clinical Pathologic Correlations (7th edition). St. Louis: Elsevier; 2017.
1 Each type of EDS has its own clinical diagnostic criteria, and genetic tests exist for most types of EDS (but not the most common type — hEDS). If a person with symptomatic joint hypermobility doesn’t meet the diagnostic criteria for any type of EDS or other condition with similar symptoms, then the hypermobility spectrum syndromes (HSD) may be a consideration.
2 less than 1/1,000,000 prevalence
3 There are no disease-specific treatments for any type of EDS, and therefore medical management is customized according to individual patient/client presentation. Treatment modalities include: pain management; use of muscle relaxants, antidepressants, and glucosamine; supplementation with vitamin C to promote wound healing; physiotherapy; cognitive behavioural therapy; and lifestyle modifications. Care often needs to be multidisciplinary, including practitioners in physiatry (physical medicine and rehabilitation), orthopaedics, rheumatology, dermatology, ophthalmology, family medicine, genetics counselling, obstetrics, physiotherapy, and occupational therapy. In Ontario, University Health Network (Toronto) has a dedicated EDS Clinic.
4 In addition to the inherent bleeding tendency of patients/clients with EDS due to tissue fragility and impaired vascular wall integrity, platelet dysfunction occurs. Evolving literature suggests that this platelet dysfunction is not confined to vEDS but is also present in other types of EDS. According to one study, nearly half of all EDS patients/clients have an elevated bleeding severity score (BSS), which, in 90% of cases, is at least partially attributable to platelet function abnormalities.
5 Such local anaesthetic resistance may be linked to the functional sequelae of altered collagen, whereby diffusion of small molecules and nutrients is affected, including oxygen delivery.
6 50% of patients/clients with EDS are able to touch their tongue to their nose (Gorlin sign), but it is unclear if lack of the lingual frenulum contributes to this sign.
7 These stones result from alteration and calcification of interpulpal vascular structures.
8 Odontogenic keratocysts are rare, non-cancerous lesions of the maxilla or mandible, which are thought to arise from the dental lamina.
9 Signs/symptoms of TMD/TMJ disorders include: pain in the temporomandibular joint(s); limitation of mouth opening; inability, or reduced ability, to chew; jaw stuck to one side; temporal area headaches; pain in mandible; inability, or reduced ability, to align teeth; pain or fullness in and around ears; and grating, clicking, or “popping” with movement of the temporomandibular joint.
10 A muscle spasm is an involuntary and forcible contraction of muscle without accompanying relaxation.
11 Molluscoid pseudotumours are spongy, small tumours found over scars and pressure points.
12 Craniocervical instability (CCI) is excessive movement of the C1 vertebra at the atlantooccipital joint. Atlantoaxial instability (AAI) is excessive C2 movement at the atlantoaxial joint. Patients/clients with EDS are susceptible to CCI and AAI.
13 Dysthesia means “abnormal sensation”. It is a generic term for a cutaneous symptom — such as burning, pruritus (itching), stinging, crawling, pain, and hypothesia — in a well-defined location and without a primary cutaneous cause. It is often caused by nerve irritation, impingement, or trauma.
14 Pneumothorax is an abnormal collection of air between the tissue layer that covers the lungs and the chest cavity. This leads to: shortness of breath; a sharp, stabbing pain that worsens with inspiration; cyanosis (bluish skin and lips); fatigue; elevated respiratory rate and pulse; and dry, hacking cough.
15 Dysautonomia is a disorder which involves failure of the sympathetic and parasympathetic parts of the autonomic nervous system (ANS). It has many manifestations, including orthostatic hypotension (i.e., drop in blood pressure when standing up) and hypertension.
16 Mast cell diseases are caused by abnormal or overly active mast cells, which are commonly found in connective tissues. When triggered, these mast cells release substances (e.g., histamine, leukotrienes, and cytokines) that can cause signs/symptoms similar to those of an allergic reaction. Other manifestations include unexplained flushing, bloating, and abdominal pain. Affected persons may feel hot even at normal room temperature. Sometimes severe inflammation can result in organ damage. Common triggers include spicy foods, alcohol, insect stings, and certain medications.
17 EDS features such as cartilage defects, craniofacial abnormalities, and increased pharyngeal collapsibility due to tissue flaccidity have been suggested to cause OSA. Collagen is a is a major component of cartilage.
18 Visceral ptosis is the sinking of the abdominal organs below their normal position. While asymptomatic in some persons, this can result in abdominal pain and/or distention, constipation or diarrhea, and nausea, amongst other signs/symptoms.
* Includes oral hygiene instruction, fitting a mouth guard, taking an impression, etc.
** Ontario Regulation 501/07 made under the Dental Hygiene Act, 1991. Invasive dental hygiene procedures are scaling teeth and root planing, including curetting surrounding tissue.